Neonatal gastrointestinal mucormycosis, a rare disease with a high mortality rate, shows a rapid progressive course in premature infants with an immature immune system. We report the case of a male neonate weighing 970 g, delivered via cesarean section at 27 weeks, as one of a pair of dizygotic twins. From the 7 th day after birth, bile was seen to drain through the orogastric tube, and paralytic ileus was noted on per forming an abdominal Xray. Thus, oral feeding was discontinued because necrotizing enterocolitis (NEC) was highly suspected. On the 9 th day after birth, a firm mass was pal pable in left upper abdominal quadrant, but no pneumatosis intestinalis was ob served on performing ab dominal Xray. Small bowel intussusception was suspected on performing abdominal ultrasonography. Based on these findings, an exploratory laparotomy was performed, and although no intussusception was found intraopera tively, we performed a partial gastrectomy and hemicolectomy due to the presence of necrotic changes and perfora tions of the stomach and colon. Post operatively, he was observed to have hypotension with persistence of hemorrhage at the surgical site. He died on the 11 th day after birth. Intraoperative histopathological examination of stomach and colon showed fungal aseptate hyphae with broad branch ing. Gastrointestinal mucormycosis was confirmed based on findings of vas cular involvement in the form of fungal hyphae and thrombosis in the transmural blood vessels. We report a case of an extremely low birth weight infant with neonatal gastrointestinal mucormycosis with an initial clinical presentation suggestive of intus susception and atypical NEC.